Liver tumors in children in the particular reference to hepatoblastoma and hepatocellular carcinoma: American Academy of pediatrics surgical section survey — 1974

Exelby, Philip R.; Filler, Robert M.; Grosfeld, Jay L.

doi:10.1016/0022-3468(75)90095-0

Cited by 306 publications

(140 citation statements)

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“…Most of the reports from the United States are case series or questionnaires of physicians. 11,[18][19][20][21] There are hospital-based case series 9,22,23 and epidemiologic analysis of hepatocellular carcinoma from the cancer registry 24 of China. The data presented here, which are based on nationwide death certificate data (NCHS) and population-based registries (SEER), provide a more complete and representative overview than can be obtained from case series.…”

Section: Discussionmentioning

confidence: 99%

“…This contrasts with previous reports, in which a stronger male predominance for both hepatoblastoma and hepatocellular carcinoma has been observed. 11,[18][19][20][21]23,24 Risk factors for hepatoblastoma are not well characterized. Hepatoblastoma has been shown to be associated with Beckwith-Weidmann syndrome 25,26 hemihypertrophy, and familial adenomatous polyposis, none of which have unique ICD-9 codes.…”

Section: Discussionmentioning

confidence: 99%

“…[8][9][10] However, the epidemiology of primary hepatic malignancies in U.S. children is not well characterized. Most available information is from institution-based case series; since a 1975 national survey of U.S. pediatric surgeons, 11 no population-based evaluation of primary liver cancer in U.S. children has been published.…”

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confidence: 99%

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Epidemiology of Primary Hepatic Malignancies in U.S. Children

et al. 2003

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The epidemiology of primary hepatic malignancies in U.S. children is poorly characterized. We analyzed the incidence, mortality, and characteristics of primary hepatic malignancies in U.S. residents less than 20 years of age. Fatal primary hepatic malignancies in persons less than 20 years of age, between 1979 and 1996, were identified using the multiple-cause-ofdeath database (National Center for Health Statistics). Histologically confirmed primary hepatic malignancies occurring between 1973 and 1997 were identified using the Surveillance, Epidemiology, and End Results (SEER) database. Between 1979 and 1996, 918 primary hepatic malignancy deaths (average, 0.7/1,000,000/year) were reported nationally among persons less than 20 years of age; rates were higher among Asians and among foreignborn children. Between 1973 and 1997, 271 primary hepatic malignancy cases were reported to SEER among persons less than 20 years of age, of which 184 (67%) and 83 (31%) were hepatoblastoma and hepatocellular carcinoma, respectively. Among children less than 5 years of age, hepatoblastoma accounted for 91% of primary hepatic malignancy cases, whereas among those 15 to 19 years of age, hepatocellular carcinoma accounted for 87% of cases. Five-year survival for hepatoblastoma was 52%, compared with 18% for hepatocellular carcinoma. In the SEER sites, between 1973 and 1977 and 1993 and 1997, hepatoblastoma rates increased (0.6 to 1.2/1,000,000, respectively), while hepatocellular carcinoma rates decreased (0.45 to 0.29/1,000,000, respectively). In conclusion, histologically confirmed hepatocellular carcinoma was reported in children less than 5 years of age, also, where hepatoblastoma is the predominant primary hepatic malignancy. Hepatocellular carcinoma has worse survival rates than hepatoblastoma, and its incidence has not increased. Better maintenance of databases may provide information about associated factors behind this unexpected occurrence. (HEPATOLOGY 2003;38:560-566.) P rimary hepatic malignancies are rare in children. 1

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Epidemiology of Primary Hepatic Malignancies in U.S. Children

et al. 2003

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“…Subsequently, a 1974 survey of the Surgical Section of the American Academy of Pediatrics reported survival rates of 35% (45/129) for HEP and 13% (12/98) for HCC. 6 Importantly, no HCC or HEP patient survived who did not undergo resection. Thus, it became apparent that resection was required to cure malignant liver tumors in children.…”

Section: Discussionmentioning

confidence: 99%

Resection, including transplantation, for hepatoblastoma and hepatocellular carcinoma: Impact on survival

Tagge

Reyes

et al. 1992

Journal of Pediatric Surgery

118

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Long-term survival in children with primary hepatic malignancies can not be expected without complete tumor resection. In the last ten years we have treated 21 children with hepatocellular carcinoma (HCC) and 21 children with hepatoblastoma (HEP), with tumor extirpation our surgical goal. Operative treatment included partial hepatectomy ([PH] 20), either primary (10) or delayed (following chemotherapy) (10), total hepatectomy and orthotopic liver transplantation ([OLT] 13), or upper abdominal exenteration and multiple organ transplantation (2). Two patients had both PH and subsequent total hepatectomy and OLT. Overall survival was 48% (20/42), with 9 patients dying of progressive disease prior to removal of their tumor. HEP patient survival was 67% (14/21), including 2 of 6 who underwent primary PH, 7 of 8 who had delayed PH, and 5 of 6 who underwent OLT. Survival for the children with HCC was 29% (6/21), including 1 of 4 after primary PH, 1 of 2 following delayed PH, 3 of 7 following OLT, and 1 of 2 after exenteration and multiple organ transplantation. Preoperative chemotherapy facilitated removal of 10 initially unresectable tumors (8 HEP, 2 HCC) at a second-look procedure. Total hepatectomy and OLT markedly improved survival in patients with disease unresectable by standard methods. Partial hepatectomy, either primary or delayed, should be attempted in all children with hepatic malignancies. Total hepatectomy and OLT appears to be a viable adjunct in the treatment of childhood malignancies, and should be used for otherwise unresectable tumors as part of a carefully planned protocol. INDEX WORDSHepatoblastoma; hepatocellular carcinoma; liver transplantation Childhood liver tumors continue to have a poor prognosis despite increasingly sophisticated diagnostic methods, aggressive surgical techniques to achieve complete resection, and more effective systemic chemotherapy. Total surgical removal remains the mainstay of treatment, although there are rare reports of long-term survivors achieved with chemotherapy alone. Preoperative chemotherapy has increased the number of patients undergoing potentially curable resections. 1 However, a large proportion of children, especially with hepatocellular carcinoma (HCC), are not resectable by standard methods. Total hepatectomy and orthotopic liver transplantation (OLT), the only alternative for unresectable tumors, is still controversial due to the high incidence of tumor recurrence. 2Address reprint requests to Edward P. Tagge, MD,

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“…It shows a particularly high prevalence in young children because about 70% of hepatoblastomas are diagnosed during the first 2 years of age. 1 The molecular mechanisms involved in the pathogenesis and development of hepatoblastoma are totally unknown. The tumor is commonly associated with loss of heterozygosity (LOH) at chromosome 11p15.5.…”

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Analysis of CDKN2A, CDKN2B, CDKN2C, and cyclin Ds gene status in hepatoblastoma

et al. 1998

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The status and the expression of cyclin-dependent kinase inhibitor A (CDKN2A) family genes, named CDKN2A, CDKN2B, and CDKN2C and of cyclin Ds (D1, D2, and D3) genes were investigated in 14 cases of human hepatoblastomas. These genes were selected because: 1) CDKN2A and CDKN2B are very frequently inactivated in human cancers; 2) cyclin Ds are overexpressed in several tumors and 3) CDKN2A is posttranscriptionally silenced in hepatocellular carcinomas. Structural analysis of the CDKN2A, CDKN2B, and CDKN2C genes in hepatoblastoma cases showed the absence of deletions and/or point mutations. Moreover, a detailed investigation of loss of heterozygosity at 9p21 and 1p32 (the chromosomal regions where CDKN2A genes are located) rules out the possible loss of one allele. Messenger RNA (mRNA) analysis showed that CDKN2C is expressed in all hepatoblastoma samples studied, while both CDKN2A and CDKN2B genes are not transcribed in the cancer specimens as well as in the matched normal liver tissues. Interestingly, an alternative mRNA expressed by the CDKN2A gene (␤-transcript) is detectable in 100% of the samples investigated. The analysis of cyclin D genes expression revealed that cyclin D1 is highly transcribed in normal hepatic tissue while cyclin D2 or D3 genes were extensively expressed in the matched transformed samples. Investigation at protein level confirmed the data obtained on RNA analysis. Indeed, p16 INK4A and p15 INK4B (products of expression of CDKN2A and CDKN2B respectively) were not observable while p18 INK4C (which is codified by CDKN2C) was clearly detectable in the samples analyzed. Moreover, a noticeable decrease of cyclin D1 content and increase of cyclin D3 level were observable in tumor tissues versus normal counterparts. Our findings demonstrated the following: 1) CDKN2A, CDKN2B, and CDKN2C genes are structurally unmodified in human hepatoblastoma, and 2) CDKN2A (␣-transcript) and CDKN2B are transcriptionally silenced in normal liver whereas CDKN2A (␤-transcript) and CDKN2C were clearly expressed. Finally, a clear shift in cyclin D type expression was observable during malignant transformation. These results show that CDKN2A gene family alterations are not involved in hepatoblastoma development, whereas changes in cyclin D types might play a role in this type of tumor. Furthermore, a highly regulated expression of CDKN2A seems to occur in normal hepatic tissue. (HEPATOLOGY 1998;27:989-995.)

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Liver tumors in children in the particular reference to hepatoblastoma and hepatocellular carcinoma: American Academy of pediatrics surgical section survey — 1974

Cited by 306 publications

References 0 publications

Epidemiology of Primary Hepatic Malignancies in U.S. Children

Epidemiology of Primary Hepatic Malignancies in U.S. Children

Resection, including transplantation, for hepatoblastoma and hepatocellular carcinoma: Impact on survival

Analysis of CDKN2A, CDKN2B, CDKN2C, and cyclin Ds gene status in hepatoblastoma

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